CEP250 is a core centrosomal protein localized to the proximal ends of centrioles where it contributes to centrosome-centrosome cohesion during interphase of the cell cycle. Centrioles are essential for the formation of centrosomes and cilia (e.g., motile cilia or non-motile cilia). Thus, the compounds that modulate CEP250 may be useful in the binding stabilization, or modulation of the activity of one or more components of the centrosome or cilia. These compounds may also be used to modulate signal transduction pathways associated with CEP250, including, but not limited to, Hedgehog, Wnt, PDGFRalpha, and integrin signaling, and the treatment of diseases or disorders related to centrosome aberrations (e.g., cancer or ciliopathies) or Hedgehog, Wnt, PDGFRalpha, or integrin signaling.
The present invention is related to compounds that modulate the activity of of target proteins such as CEP250 and, therefore, may be useful in the treatment of diseases and disorders such as cancer, ciliopathies, or infections.